In patients born with congenital heart disease, dilatation of the aorta is a frequent feature at presentation and during follow-up after surgical intervention. This review provides an overview of the pathologies associated with aortopathy, and discusses the current knowledge on pathophysiology, evolution, and treatment guidelines of the aortic disease associated with congenital heart defects.

Background: Tetralogy of Fallot (TOF) with subarterial ventricular septal defect (VSD) is more common among Asians than Caucasians. Compared with the regular subaortic VSD postoperative right ventricular outflow obstruction is more common because of the sub-pulmonary extension of the defect. The objective of this study is to analyze the surgical implications and outcomes of patients with TOF - subarterial VSD in the current era.
Patients and Methods: In all, 539 consecutive operated patients with TOF from May 2005 to September 2012 were retrospectively reviewed. Eighty-five patients had subarterial VSD. Seventy-nine of these underwent intracardiac repair. Preoperative clinical, echocardiographic features, operative and postoperative variables were assessed.
Results: The median age at surgery was 6 years and the median weight was 14 kilograms. The male to female ratio was 1.7:1. TOF with subarterial VSD was associated with frequent use of transannular patch (74.6%). The early mortality was 2.5%. Follow up was 92% complete with a mean duration of 20 months with actuarial survival of 97.3% at 5 years. Two patients required reoperation for significant right ventricular outflow tract obstruction (RVOTO) at one year and three years, respectively.
Conclusions: Intra cardiac repair for TOF with subarterial VSD has low perioperative mortality and morbidity. Transannular patch augmentation of the right ventricular outflow tract (RVOT) is required in a significant proportion of these patients. Precise suturing of the VSD patch, adequate infundibular resection and lower threshold for a transannular patch placement ensures a smooth early postoperative recovery.

Background: Training of postgraduate students has traditionally been done in person in a hospital setting with hands-on training with each faculty member imparting knowledge to 2 to 4 students per year. Supplementing their practical education with online instruction could make a significant difference in standardizing pediatric cardiology education in India.
Objective: To present the rationale, methods and survey results of a live e-Teaching methodology implemented for Pediatric cardiology trainees in association with the National Board of Examinations, India.
Materials and Methods: Between March 2010 and March 2014, 310 e-classes were conducted in the Pediatric cardiac sciences by 24 e-teachers. Content of the e-Learning program was based on a 2-year pediatric cardiology curriculum and included twice-weekly live online video training sessions, a library of recorded sessions and online test quizzes for the students. A total of 231 students accessed the program at various times over the 4-year period.
Results: In our study, requests for access to the e-lectures increased from 10/year the first year to 100/year by the fourth year with feedback surveys conveying a high satisfaction level from the students and a high need for this knowledge. The advantages of virtual live e-Learning included the fact that one teacher can teach multiple students in multiple geographic locations at the same time, obviating the issue of quality teacher shortage and the same content can be disseminated to all students undergoing specialist training so there is a national consensus on diagnostic and management approach among all trainees. Additionally, the e-classes can be recorded and replayed so they can be viewed repeatedly by the same group or new trainees.
Conclusion: This is the first sustained use of e-Teaching in a medical super-specialty in India. We believe that e-Teaching is an innovative solution that can be applied, not just to Pediatric Cardiology as we have done, but to all branches of specialist and super-specialist medical training in India and globally.

Aim: Junctional ectopic tachycardia (JET) is a postoperative complication with a mortality rate of up to 14% after surgery for congenital heart disease. This study evaluated the risk factors of JET and explored the association of postoperative temporary third degree atrioventricular (AV) block and the occurrence of JET.
Materials and Methods: Data were collected retrospectively from 1158 patients who underwent surgery for congenital heart disease.
Results: The overall incidence of JET was 2.8%. Temporary third degree AV block occurred in 1.6% of cases. Permanent third degree AV block requiring pacemaker implantation occurred in 1% of cases. In all, 56% of patients with JET had temporary AV block (P < 0.001), whereas no case of postoperative JET was reported in patients with permanent AV block (P = 0.56). temporary third degree AV block did not suffer from JET.
Conclusions: A correlation between temporary third degree AV block and postoperative JET could be observed. The risk factors identified for JET include younger age groups at the time of surgery, longer aortic cross clamping time and surgical procedures in proximity to the AV node.

A wide atrial communication is important to maintain hemodynamics in certain forms of congenital and acquired heart defects. In comparison to balloon septostomy or blade septostomy, atrial septal stenting provides a controlled, predictable, and long-lasting atrial communication. It often needs a prior Brockenbrough needle septal puncture to obtain a stable stent position. A stent deployed across a previously dilated and stretched oval foramen or tunnel form of oval foramen carries higher risk of embolization. This review provides technical tips to achieve a safe atrial septal stenting. Even though this is a "How to do it article," an initial discussion about the indications for atrial septal stenting is vital as the resultant size of the atrial septal communication should be tailored for each indication.

We describe a 7-week-old female infant who presented with refractory supraventricular tachycardia (SVT). During amiodarone infusion, she developed hypotension and cardiac arrest requiring extracorporeal membrane oxygenation (ECMO) support. After successful control of SVT using procainamide infusion, she was weaned from ECMO and discharged home on oral flecainide. We conclude that infants with acidosis, ventricular dysfunction, and prolonged refractory SVT may poorly tolerate intravenous amiodarone.

Aim: The present study was undertaken to assess the accuracy of pediatric echocardiograms done at non-tertiary centers and to evaluate the relationship of inaccurate interpretations with age, echocardiogram performer and complexity of congenital heart disease (CHD).
Materials and Methods: The echocardiogram reports of 182 consecutive children with CHD (5 days-16 years) who were evaluated at a non-tertiary center and subsequently referred to our center were reviewed. Age of the child at echocardiogram, echocardiogram performer and complexity of CHD were noted. These reports were compared with echocardiogram done at our center. Discrepancies were noted and categorized. To assess our own error rate, we compared our echocardiogram reports with the findings obtained during surgery (n = 172), CT scan (n = 9) or cardiac catheterization reports (n = 1).
Results: Most of the children at the non-tertiary center (92%) underwent echocardiogram by personnel other than a pediatric cardiologist. Overall, diagnostic errors were found in 69/182 (38%) children. Moderate and major discrepancies affecting the final management were found in 42/182 (23%) children. Discrepancies were higher when the echocardiogram was done by personnel other than pediatric cardiologist (P < 0.01) and with moderate and high complexity lesions (P = 0.0001). There was no significant difference in proportion of these discrepancies in children ≤ 1 year vs. >1 year of age.
Conclusions: A significant number of pediatric echocardiograms done at non-tertiary centers had discrepancies that affected the management of these children. More discrepancies were seen when the echocardiogram performer was not a pediatric cardiologist and with complex CHD.

Abernethy syndrome (congenital extrahepatic portosystemic shunt (CEPS II)) as an etiology of hepatopulmonary syndrome (HPS) is uncommon. The severe hypoxemia and its consequences become incapacitating for the patient. Early shunt closure resolves hypoxemia and clinical symptomatology and prevents irreversible changes in pulmonary vasculature.

This is the first report of the use of veno-venous extracorporeal membrane oxygenation in a neonate with severe Ebstein's anomaly. The report suggests the use of veno-venous extracorporeal membrane oxygenation in the immediate neonatal period may be a useful therapy in severe Ebstein's anomaly. By providing adequate oxygenation independent of the patient's native pulmonary blood flow, veno-venous extracorporeal membrane oxygenation allows the pulmonary vascular resistance to decrease and may promote right ventricular recovery.

Partnership in healthcare: What can the west learn from the delivery of pediatric cardiac care in low- and middle-income countriesSeshadri Balaji, Raman Krishna KumarJanuary-April 2015, 8(1):1-3DOI:10.4103/0974-2069.149509 PMID:25684879

The 5 th Congress of Asia Pacific Pediatric Cardiac Society was held in New Delhi from 6-9 March 2014. This article describes the journey of preparing and hosting one of the largest international events in the specialty of Pediatric Cardiac Care ever held in India. A total of 938 delegates, including 400 from outside India, participated. The scientific program was inclusive keeping in mind the diverse background of delegates from the member nations. Large numbers of research papers were presented, mostly by fellows in training.

In an adolescent girl with coexistent valvar pulmonary stenosis (PS) and muscular ventricular septal defect (VSD) causing right to left shunt and severe cyanosis, a series of complications were encountered during transcatheter intervention. After balloon pulmonary valvotomy (BPV) and device closure of the VSD, dynamic infundibular hypercontractility elevated the right ventricular (RV) systolic pressures leading to embolization of the device into the left ventricle. During retrieval of the device from the left ventricular outflow tract (LVOT), there was injury to the atrioventricular nodal tissue resulting in transient complete heart block. Even though the device was successfully retrieved out from the right femoral arterial access, there was a retroperitoneal hematoma causing severe anemia. A combination of dynamic infundibular obstruction, intravascular volume depletion caused by hemorrhage, low oxygen carrying capacity due to anemia, and inotropes resulted in a life-threatening hypercyanotic spell. Once this combination of suicidal hypercontractile right ventricle, anemia, and dehydration was recognized; management of the pathophysiology resulted in recovery of the patient. On a subsequent day, device closure of the VSD was complicated by unstable device position, but was finally achieved by an innovative device stabilization technique. We propose to explain the basis of hemodynamic and procedural complications, their recognition, and management. During preparatory stage before interventions in patients with multiple defects, which are linked by strong hemodynamic interdependence, meticulous planning should be done and multiple untoward events should be foreseen. While a few complications were completely unanticipated, some others could have been predicted.

We report a case of Uhl's anomaly in a 3-week-old infant that underwent central shunt placement, patent duct us arteriosus and main pulmonary artery ligation. The infant presented with room air saturation of 43%, dilated right ventricle with decreased function and dilated right atrium. Diagnosis was established with a myocardial biopsy.

A 3.5-year-old girl underwent transcatheter closure of patent ductus arteriosus in early infancy during which time her secundum atrial septal defect (ASD) was left alone. When she came for elective closure of ASD, she was found to have bilaterally blocked femoral veins. The defect was successfully closed with an Amplatzer septal occluder (ASO; St. Jude Medical, Plymouth, MN, USA) using a hybrid approach via a sub-mammary mini-thoracotomy incision without using cardiopulmonary bypass. At the end of 1-year follow-up, the child is asymptomatic with device in a stable position without any residual shunt.

Challenges in the management of congenital heart disease in Vietnam: A single center experienceVu Minh Phuc, Do Nguyen Tin, Do Thi Cam GiangJanuary-April 2015, 8(1):44-46DOI:10.4103/0974-2069.149517 PMID:25684886

Vietnam, in Asia, is a low middle-income country with a relatively large population to cater to. Not many know about Vietnam, or its healthcare sector especially the field of pediatric cardiology and congenital heart disease. In contrast to the developed world, congenital heart disease (CHD) is not diagnosed early. Since most of the patients visit the hospital only in later stages of the disease there are many complications during the operation and post-operatively. But during the past 5 years (from 2009), there has been major improvement in the treatment of CHD, both by intervention and surgery. At present, all kinds of CHD, both simple and complex are being successfully treated in our country. Today in Vietnam, all children under 6 years of age have health insurance coverage, under which almost all operations and catheter interventions are done free in government hospitals. It is helping many patients, especially those from the poor socioeconomic background. However, the present infrastructure is inadequate and a long waiting list has accumulated for treatment of CHD.

An eleven-year-old boy expectorated a foreign body in cough that was identified as the prosthetic graft used for a Blalock-Taussig shunt. The shunt procedure was done 10 years earlier, and a definitive repair for tetralogy of Fallot was done a year later. He had no other symptoms, and a computed tomography (CT) angiogram did not reveal any other significant anomaly. The reason for this extremely rare event is unclear.

Cardiac injuries during repeat sternotomy are rare. While undergoing debridement for chronic osteomyelitis (post arterial septal defect closure), a 4-year-old girl sustained significant right ventricular (RV) injury. Bleeding from the RV was controlled by packing the injury site, which helped in maintaining stable hemodynamics till arrangements were made for instituting cardiopulmonary bypass (CPB). Since the femoral artery was very small and unsuitable for direct cannulation, a polytetrafluoroethylene (PTFE) graft sutured end-to-side to the femoral artery was used for establishing CPB. The injury was successfully repaired.

The Scimitar syndrome is a complex association of cardiovascular and bronchopulmonary abnormalities, with the main feature a partial or total anomalous right pulmonary venous drainage to the inferior vena cava. A number of cases that lack of all the features of the typical syndrome have been described as Scimitar variant, but the incidence is rare. Familial occurrence is exceptional and limited to few cases in literature. We report two sibling diagnosed with an uncommon variant of the Scimitar syndrome.

Ventricular septal defect (VSD) in Tetralogy of Fallot (TOF) is usually large and non-restrictive with equalization of right and left ventricular pressures. Restrictive VSD in TOF is rare. We present an unusual case of TOF with restriction to VSD caused by accessory tricuspid valve tissue that varied with respiration.